Table 2

Serious adverse events listed in descending order of incidence by patient group*

Preferred term*
n (IR/100 PY) (95% CI)		FCAS
(n=42)		MWS
(n=169)		NOMID
(n=32)		All CAPS
(n=243)		Other†
(n=42)		All
(N=285)
Any SAE4 (2.6)
(0.7 to 6.6)		100 (15.4)
(12.6 to 18.8)		24 (21.1)
(13.5 to 31.4)		128 (13.9)
(11.6 to 16.6)		27 (19.3)
(12.73 to 28.11)		155 (14.7)
(12.4 to 17.2)
Pneumonia0
(0.0 to 2.4)		5 (0.8)
(0.3 to 1.8)		0
(0.0 to 3.2)		5 (0.54)
(0.2 to 1.3)		2 (1.43)
(0.17 to 5.17)		7 (0.7)
(0.3 to 1.4)
Pancreatitis0
(0.0 to 2.4)		6 (0.9)
(0.3 to 2.0)		0
(0.0 to 3.2)		6 (0.7)
(0.2 to 1.4)		0
(0.0 to 2.7)		6 (0.6)
(0.2 to 1.2)
Headache0
(0.0 to 2.4)		1 (0.2)
(0.0 to 0.9)		3 (2.6)
(0.5 to 7.7)		4 (0.4)
(0.1 to 1.1)		0
(0.0 to 2.7)		4 (0.4)
(0.1 to 1.0)
Tonsillitis1 (0.6)
(0.0 to 3.6)		2 (0.3)
(0.0 to 1.1)		0
(0.0 to 3.2)		3 (0.3)
(0.1 to 1.0)		1 (0.7)
(0.0 to 4.0)		4 (0.4)
(0.1 to 1.0)
Urinary tract infection0
(0.0 to 2.4)		3 (0.5)
(0.1 to 1.4)		1 (0.9)
(0.0 to 4.9)		4 (0.4)
(0.1 to 1.1)		0
(0.0 to 2.7)		4 (0.4)
(0.1 to 1.0)
Angina pectoris0
(0.0 to 2.4)		3 (0.5)
(0.1 to 1.4)		0
(0.0 to 3.2)		3 (0.3)
(0.1 to 1.0)		0
(0.0 to 2.7)		3 (0.3)
(0.1 to 0.8)
Bronchitis0
(0.0 to 2.4)		3 (0.5)
(0.1 to 1.4)		0
(0.0 to 3.2)		3 (0.3)
(0.1 to 1.0)		0
(0.0 to 2.7)		3 (0.3)
(0.1 to 0.8)
Depression0
(0.0 to 2.4)		2 (0.3)
(0.0 to 1.1)		1 (0.9)
(0.0 to 4.9)		3 (0.3)
(0.1 to 1.0)		0
(0.0 to 2.7)		3 (0.3)
(0.1 to 0.8)
Intracranial pressure increased0
(0.0 to 2.4)		0
(0.0 to 0.6)		2 (1.8)
(0.2 to 6.4)		2 (0.2)
(0.0 to 0.8)		1 (0.7)
(0.0 to 4.0)		3 (0.3)
(0.1 to 0.8)
Rectal cancer0
(0.0 to 2.4)		3 (0.5)
(0.1 to 1.4)		0
(0.0 to 3.2)		3 (0.3)
(0.1 to 1.0)		0
(0.0 to 2.7)		3 (0.3)
(0.1 to 0.8)
Vaccination site inflammation0
(0.0 to 2.4)		3 (0.5)
(0.1 to 1.4)		0
(0.0 to 3.2)		3 (0.3)
(0.1 to 1.0)		0
(0.0 to 2.7)		3 (0.3)
(0.1 to 0.8)
Vertigo0
(0.0 to 2.4)		1 (0.2)
(0.0 to 0.9)		2 (1.8)
(0.2 to 6.4)		3 (0.3)
(0.1 to 1.0)		0
(0.0 to 2.7)		3 (0.3)
(0.1 to 0.8)
Abortion spontaneous0
(0.0 to 2.4)		2 (0.3)
(0.0 to 1.1)		0
(0.0 to 3.2)		2 (0.2)
(0.0 to 0.8)		0
(0.0 to 2.7)		2 (0.2)
(0.0 to 0.7)
Abscess0
(0.0 to 2.4)		2 (0.3)
(0.0 to 1.1)		0
(0.0 to 3.2)		2 (0.2)
(0.0 to 0.8)		0
(0.0 to 2.7)		2 (0.2)
(0.0 to 0.7)
Cellulitis0
(0.0 to 2.4)		1 (0.2)
(0.0 to 0.9)		1 (0.9)
(0.0 to 4.9)		2 (0.2)
(0.0 to 0.8)		0
(0.0 to 2.7)		2 (0.2)
(0.0 to 0.7)
Cholelithiasis0
(0.0 to 2.4)		2 (0.3)
(0.0 to 1.1)		0
(0.0 to 3.2)		2 (0.2)
(0.0 to 0.8)		0
(0.0 to 2.7)		2 (0.2)
(0.0 to 0.7)
Condition aggravated0
(0.0 to 2.4)		1 (0.2)
(0.0 to 0.9)		1 (0.9)
(0.0 to 4.9)		2 (0.2)
(0.0 to 0.8)		0
(0.0 to 2.7)		2 (0.2)
(0.0 to 0.7)
Glaucoma0
(0.0 to 2.4)		1 (0.2)
(0.0 to 0.9)		1 (0.9)
(0.0 to 4.9)		2 (0.2)
(0.0 to 0.8)		0
(0.0 to 2.7)		2 (0.2)
(0.0 to 0.7)
Knee deformity0
(0.0 to 2.4)		0
(0.0 to 0.6)		2 (1.8)
(0.2 to 6.4)		2 (0.2)
(0.0 to 0.8)		0
(0.0 to 2.7)		2 (0.2)
(0.0 to 0.7)
Lower respiratory tract infection0
(0.0 to 2.4)		1 (0.2)
(0.0 to 0.9)		1 (0.9)
(0.0 to 4.9)		2 (0.2)
(0.0 to 0.8)		0
(0.0 to 2.7)		2 (0.2)
(0.0 to 0.7)
Meningitis0
(0.0 to 2.4)		0
(0.0 to 0.6)		2 (1.8)
(0.2 to 6.4)		2 (0.2)
(0.0 to 0.8)		0
(0.0 to 2.7)		2 (0.2)
(0.0 to 0.7)
Perirectal abscess0
(0.0 to 2.4)		2 (0.3)
(0.0 to 1.1)		0
(0.0 to 3.2)		2 (0.2)
(0.0 to 0.8)		0
(0.0 to 2.7)		2 (0.2)
(0.0 to 0.7)
Pyrexia0
(0.0 to 2.4)		1 (0.2)
(0.0 to 0.9)		1 (0.9)
(0.0 to 4.9)		2 (0.2)
(0.0 to 0.8)		0
(0.0 to 2.7)		2 (0.2)
(0.0 to 0.7)

  • *Number of events ≥2 in all registry patients; see online supplemental table S8 for number of events=1 in all registry patients.

  • †Other includes atypical CAPS (n=18) and non-CAPS diagnoses (n=24); non-CAPS diagnoses include systemic juvenile idiopathic arthritis (n=8), unspecified autoinflammatory syndromes (n=5), familial Mediterranean fever (n=3), mevalonate kinase deficiency (n=2), adult-onset Still’s disease (n=2), tumour necrosis factor receptor-associated periodic syndrome (n=1), Erdheim-Chester disease (n=1), Blau syndrome (n=1) and granulomatosis with polyangiitis (n=1).

  • CAPS, cryopyrin-associated periodic syndromes; FCAS, familial cold autoinflammatory syndrome; IR, incidence rate; MWS, Muckle-Wells syndrome; n, number of events; NOMID, neonatal-onset multisystem inflammatory disease; PY, patient-years; SAE, serious adverse event.